Antiphospholipid syndrome is characterized by venous and arterial thrombosis, recurrent pregnancy loss and the presence of the lupus anticoagulant, anticardiolipin antibodies or both. Antiphospholipid syndrome may occur as a primary disease or in patients with systemic lupus erythematosus or other autoimmune, infectious or neoplastic disorders. In this paper we report a 29-year-old Saudi female, a known case of antiphospholipid syndrome, presented with complaints of fever, breathlessness and generalized fatigue. Further investigations confirmed her as a case of myeloblastic leukemia (M1, French-American-British classification). Acute myeloblastic leukemia is not described to be associated with primary antiphospholipid syndrome in the literature to date. This is the first case report of such association.